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Bulletin of Experimental Biology and... May 2018The diagnostic potentialities of complex immunochemical analysis of the serum and daily urine were evaluated in 118 patients with multiple myeloma. In 95 patients, we...
The diagnostic potentialities of complex immunochemical analysis of the serum and daily urine were evaluated in 118 patients with multiple myeloma. In 95 patients, we observed secretion of monoclonal intact immunoglobulins with heavy chains G (N=69), A (N=19), and M (N=4) and biclonal secretion of paraproteins G and A (N=3). Bence-Jones protein was detected in the sera and daily urine of 16 patients and Bence-Jones proteinuria alone was detected in 3 patients. The diagnostic sensitivity of serum immunoelectrophoresis in multiple myeloma is 94.1%. Analysis of paraproteinuria is particularly important in Bence-Jones myeloma, when paraprotein excretion may be not associated with paraproteinemia. Complex study by immunoelectrophoretic and immunoturbidimetric methods in multiple myeloma increases the diagnostic sensitivity to 99.2%.
Topics: Adult; Aged; Aged, 80 and over; Bence Jones Protein; Female; Humans; Immunoelectrophoresis; Male; Middle Aged; Multiple Myeloma; Paraproteinemias
PubMed: 29797132
DOI: 10.1007/s10517-018-4105-y -
Journal of Clinical Pathology Nov 2023Monoclonal gammopathy is a spectrum of disorders characterised by clonal proliferation of plasma cells or lymphocytes, which produce abnormal immunoglobulin or its...
Monoclonal gammopathy is a spectrum of disorders characterised by clonal proliferation of plasma cells or lymphocytes, which produce abnormal immunoglobulin or its components (monoclonal proteins). Monoclonal gammopathies are often categorised as low-tumour-burden diseases (eg, amyloid light chain (AL) amyloidosis), premalignant disorders (such as monoclonal gammopathy of undetermined significance and smouldering multiple myeloma), and malignancies (eg, multiple myeloma and Waldenström's macroglobulinaemia). Such diversity of concentration and structure makes monoclonal protein a challenging clonal marker. This article provides an overview on initial laboratory testing of monoclonal gammopathy to guide clinicians and laboratory professionals in the selection and interpretation of appropriate investigations.
Topics: Humans; Monoclonal Gammopathy of Undetermined Significance; Multiple Myeloma; Paraproteinemias; Precancerous Conditions
PubMed: 37604683
DOI: 10.1136/jcp-2023-208774 -
Annals of Hematology Aug 2017Monoclonal gammopathy of undetermined significance (MGUS) is a clonal plasma cell disorder and precursor disease to multiple myeloma and other related cancers. While... (Review)
Review
Monoclonal gammopathy of undetermined significance (MGUS) is a clonal plasma cell disorder and precursor disease to multiple myeloma and other related cancers. While MGUS is considered a benign disorder, with a low risk of disease progression, patients have altered bone microarchitecture and an increased risk of bone fracture. In addition, alterations in immune function are regularly found to correlate with disease activity. Vitamin D, an important hormone for bone and immune health, is commonly deficient in multiple myeloma patients. However, vitamin D deficiency is also prevalent in the general population. The purpose of this review is to highlight the current understanding of vitamin D in health and disease and to parallel this with a review of the abnormalities found in plasma cell dyscrasias. While some consensus statements have advocated for vitamin D testing and routine supplementation in MGUS, there is no clear standard of care approach and clinical practice patterns vary. Further research is needed to better understand how vitamin D influences outcomes in MGUS patients.
Topics: Disease Progression; Humans; Immune System; Models, Biological; Monoclonal Gammopathy of Undetermined Significance; Multiple Myeloma; Paraproteinemias; Risk Factors; Vitamin D; Vitamin D Deficiency
PubMed: 28502031
DOI: 10.1007/s00277-017-3016-8 -
European Journal of Medical Research Jul 2021This study aimed to analyze the clinicopathological characteristics of patients with paraproteinemia and renal damage.
BACKGROUND
This study aimed to analyze the clinicopathological characteristics of patients with paraproteinemia and renal damage.
METHODS
Ninety-six patients from 2014 to 2018 with paraproteinemia and renal damage were enrolled and the clinical data, renal pathology, treatment and prognosis data were collected.
RESULTS
A total of 96 patients (54 male and 42 female), accounting for 2.7% of all renal biopsies, were enrolled in this study. Among them, 42 were monoclonal gammopathy of renal significance (MGRS), 21 were renal monotypic immunoglobulin alone (renal monoIg), and 19 were monoclonal gammopathy of undetermined significance (MGUS). Individuals with multiple myeloma (MM) accounted for the fewest number of patients (n = 14). In the MGRS group, the main diseases were amyloidosis (n = 25) and cryoglobulinemic glomerulonephritis (n = 7), while in the MM group, the main diseases were cast nephropathy (n = 9) and light chain deposit disease (n = 3). In the MGUS group, it was mainly IgA nephropathy (IgAN, n = 10) and idiopathic membranous nephropathy (n = 5); while in the renal monoIg group, most of the cases were IgAN (n = 19). Chemotherapy was mainly administered to patients in the MM group, while immunosuppression therapy was mostly administered to patients in the renal monoIg group. Most patients with renal monoIg exhibited a major response, followed by the patients with MGUS and MGRS, while most of the patients with MM had a partial response but none had a major response. Approximately more than half (57.1%) of the patients with MM progressed to end-stage renal disease (ESRD), followed by MGRS (33.3%); however, the mortality rate was low in both the MGRS and MM groups. The survival analysis reviewed that serum creatinine, hemoglobin levels, and the serum κ/λ ratio were independent risk factors for ESRD in patients with MGRS.
CONCLUSIONS
The clinicopathological changes in patients with MGRS were between those in patients with MM and MGUS. The treatment for MGRS and MM was more intensive, and the overall mortality rate was low. Both MGUS and renal monoIg alone exhibited slighter clinicopathological features than MGRS and MM, and the treatment was focused mostly on primary renal diseases.
Topics: Adult; Biopsy; China; Female; Humans; Incidence; Kidney; Kidney Diseases; Male; Middle Aged; Paraproteinemias; Prognosis
PubMed: 34217367
DOI: 10.1186/s40001-021-00538-2 -
Advances in Anatomic Pathology Mar 2015Monoclonal gammopathy is produced by neoplastic or non-neoplastic expansion of a clone of plasma cells or B lymphocytes. Monoclonal gammopathy of unknown significance is... (Review)
Review
Monoclonal gammopathy is produced by neoplastic or non-neoplastic expansion of a clone of plasma cells or B lymphocytes. Monoclonal gammopathy of unknown significance is characterized by low levels of the monoclonal protein and a relatively small population of clonal lymphocytes or plasma cells in the bone marrow. In these cases, the patient is asymptomatic with no evidence of overt myeloma or lymphoma. The abnormal serum protein may be present as a complete immunoglobulin molecule or may consist of ≥1 of its components such as light chains or heavy chains. These proteins may cause a variety of diseases in various tissues and organs, of which the kidney appears to be the most vulnerable. Renal involvement in monoclonal gammopathy may occur as part of a generalized disease such as amyloidosis, immunoglobulin deposition disease, and cryoglobulinemia. In addition, there may be evidence of kidney damage by processes which are renal specific. These include light chain proximal tubulopathy, light chain cast nephropathy, and a variety of glomerulopathies encompassing a wide spectrum of disease patterns.
Topics: Amyloidosis; Humans; Immunoglobulins; Kidney; Kidney Diseases; Paraproteinemias
PubMed: 25664947
DOI: 10.1097/PAP.0000000000000056 -
Nephrology, Dialysis, Transplantation :... Oct 2023Light chain proximal tubulopathy (LCPT) is a rare form of paraprotein-related disease, occurring in two main histopathological forms: crystalline and non-crystalline....
BACKGROUND
Light chain proximal tubulopathy (LCPT) is a rare form of paraprotein-related disease, occurring in two main histopathological forms: crystalline and non-crystalline. The clinicopathological features, treatment strategies and outcomes, especially of the non-crystalline form, are not well described.
METHODS
We conducted a single-centre retrospective case series of 12 LCPT patients, 5 crystalline and 7 non-crystalline, between 2005 and 2021.
RESULTS
The median age was 69.5 years (range 47-80). Ten patients presented with CKD and significant proteinuria (median estimated glomerular filtration rate of 43.5 ml/min/1.73 m2; urine protein:creatinine ratio 328 mg/mmol). Only six patients had known haematological disease at the time of renal biopsy. Multiple myeloma (MM) was diagnosed in seven patients cases and monoclonal gammopathy of renal significance (MGRS) in five patients. A clone was detected in all cases combining serum/urine electrophoresis and free light chain (LC) assays. Crystalline and non-crystalline variants had similar clinical presentations. For the non-crystalline variant, a diagnosis was reached based on a combination of CKD without another cause, haematological workup, LC restriction on immunofluorescence and abnormalities on electron microscopy (EM). Nine of 12 patients received clone-directed treatment. Patients who achieved haematological response (including all non-crystalline LCPT) had improved renal outcomes over a median follow-up of 79 months.
CONCLUSIONS
The non-crystalline variant may go unrecognised because of its subtle histopathological features and requires EM to distinguish it from 'excessive LC resorption without tubular injury'. Clone-directed treatment with good haematological response improves renal outcomes in both variants but limited data exist in MGRS. Multicentre prospective studies are needed to better define the clinicopathological characteristics associated with poor outcomes and optimize treatment strategies in patients with MGRS.
Topics: Humans; Middle Aged; Aged; Aged, 80 and over; Retrospective Studies; Kidney Diseases; Kidney; Multiple Myeloma; Immunoglobulin Light Chains; Renal Insufficiency, Chronic; Paraproteinemias
PubMed: 37120733
DOI: 10.1093/ndt/gfad085 -
International Journal of Dermatology Aug 2014Scleromyxedema is a rare generalized form of lichen myxedematosus, a chronic cutaneous mucinosis of unknown etiology usually associated with a monoclonal gammopathy and...
BACKGROUND
Scleromyxedema is a rare generalized form of lichen myxedematosus, a chronic cutaneous mucinosis of unknown etiology usually associated with a monoclonal gammopathy and underlying systemic disorders. It is characterized by the presence of lichenoid papules and diffuse indurations of the skin. Histologically, mucin deposits are observed in the dermis as variable degrees of fibrosis. Numerous treatment modalities have been reported as producing partial or inconsistent responses associated with significant adverse effects.
METHODS
We report an unusual case of scleromyxedema not associated with monoclonal gammopathy in a young patient who was treated with thalidomide.
RESULTS
Patient remained stable with maintenance of injuries despite treatment with thalidomide.
CONCLUSIONS
Scleromyxedema is a rare presentation for which a defined therapeutic regimen remains to be established. Treatment with thalidomide has proved to be effective in the management of these patients. We suggest that these patients should be followed up with periodic protein electrophoresis with immunofixation for a monoclonal component in blood and urine. As the therapeutic approach to scleromyxedema remains challenging and treatment is based on symptomatic presentation, further clinical studies to substantiate an effective therapeutic regimen with a positive long-term safety and risk profile are required.
Topics: Adolescent; Humans; Immunosuppressive Agents; Male; Paraproteinemias; Scleromyxedema; Thalidomide
PubMed: 24527753
DOI: 10.1111/ijd.12124 -
Current Opinion in Neurology Oct 2014This review summarizes the major recent developments in understanding of pathogenic mechanisms in inflammatory and paraproteinaemic neuropathies. (Review)
Review
PURPOSE OF REVIEW
This review summarizes the major recent developments in understanding of pathogenic mechanisms in inflammatory and paraproteinaemic neuropathies.
RECENT FINDINGS
In the inflammatory neuropathies, there has been a particular focus on antibody-mediated disease affecting the nodal/paranodal regions. Disruption of electrical integrity at these sites on the axonal membrane can cause conduction block without other electrophysiological features of demyelination, which has led to calls for a revision in the classification of axonal versus demyelinating neuropathies to include the new category of 'nodo-paranodopathies'. There has likewise been an expansion in knowledge regarding the diverse disease mechanisms of the paraproteinaemic neuropathies. These also include disease mediated by antibodies binding to peripheral nerve antigens, but additionally encompass immune complex deposition, cellular infiltration, and cytokine production.
SUMMARY
An increasing range of laboratory tests for antibodies, growth factors, and cytokines are proposed as useful in the management of inflammatory and paraproteinaemic neuropathies. Furthermore, the traditional electrodiagnostic classification into axonal and demyelinating neuropathy may not always accurately reflect the underlying disease process. Understanding how these paraclinical tests aid in identifying the underlying disease has relevance to the practising clinician both in terms of diagnosis and for the selection of rational treatments.
Topics: Autoantibodies; Guillain-Barre Syndrome; Humans; Paraproteinemias; Paraproteins; Peripheral Nervous System Diseases; Polyradiculoneuropathy, Chronic Inflammatory Demyelinating
PubMed: 25159930
DOI: 10.1097/WCO.0000000000000137 -
Revista Espanola de Patologia :... 2022The kidney is one of the organs most frequently affected by disease processes which produce monoclonal immunoglobins, therefore renal morphological and...
INTRODUCTION
The kidney is one of the organs most frequently affected by disease processes which produce monoclonal immunoglobins, therefore renal morphological and immunopathological alterations should be clearly recognized.
OBJECTIVE
To describe the pathological features of renal involvement in monoclonal gammopathies.
MATERIAL AND METHODS
A descriptive, retrospective and cross-sectional study of renal biopsies studied in a single center during a period of 14 years was carried out.
RESULTS
102 cases were included, of which 53% were male patients and the median age was 62.5 years (range 34 - 79). 97% of the biopsies were from native kidneys. The most frequent histopathological diagnosis (31.4%) was myeloma kidney, with kappa being the light chain most frequently deposited (65.6% of cases). AL amyloidosis was the second most common (29.4%) where the lambda chain predominated in 86.6%, followed by light chain deposition disease (20.6%) with the predominance of the kappa chain in 66.6%.
CONCLUSIONS
The most frequent renal involvement due to monoclonal gammopathies was myeloma kidney with deposition of kappa light chains, followed by AL lambda amyloidosis; these diseases were found more frequently in patients over 50 years of age.
Topics: Adult; Aged; Cross-Sectional Studies; Humans; Kidney Diseases; Male; Middle Aged; Multiple Myeloma; Paraproteinemias; Retrospective Studies
PubMed: 34980439
DOI: 10.1016/j.patol.2021.06.002 -
Clinical and Experimental Dermatology Apr 2022
Topics: Humans; Monoclonal Gammopathy of Undetermined Significance; Paraproteinemias; Skin
PubMed: 34905231
DOI: 10.1111/ced.15062